How to use glucocorticoids appropriately in systemic lupus erythematosus

  In order to further standardize the application of glucocorticoids in patients with SLE and benefit more patients, the CSTAR expert group has formulated an expert consensus on glucocorticoid therapy for SLE.  1. Basic principles of glucocorticoid therapy for SLE: (1) assess the severity and activity of SLE and draw up an individualized treatment plan; (2) assess whether there are relative contraindications to the use of glucocorticoids, and for patients with relative contraindications, the necessity of using glucocorticoids should be strictly evaluated according to their conditions; (3) for induction of remission and long-term maintenance therapy, the starting dose should be adequate, after which (4) For patients with hepatic impairment, it is recommended to apply prednisolone or methylprednisolone; (5) During treatment, observe the efficacy and organ function; (6) Monitor the possible complications during glucocorticoid application and adjust the treatment plan in time.  (2) The dosage of glucocorticoids for SLE treatment: (1) The usage of glucocorticoids includes systemic application (intravenous and oral) and local application (local skin topical, joint cavity injection, intraocular injection, etc.); (2) According to the needs of the disease, glucocorticoids can be administered in the morning, every other day or daily in divided doses. (3) Glucocorticoids can be divided into 4 dose ranges: small dose: prednisone 7,5mg/d or less (methylprednisolone 6mg/d or less) for maintenance treatment; medium dose: prednisone 7,5mg/d-30mg/d (methylprednisolone 6mg/d-24mg) for patients with milder symptoms; high dose. Prednisone 30mg/d-100mg/d (methylprednisolone 24mg/d-80mg), for patients with active disease; shock therapy: methylprednisolone 500-1000mg/d, intravenous drip, for 3 days, for critical patients.  3. Definition of SLE severity and lupus crisis (1) Light SLE: refers to SLE with clear diagnosis and no involvement of important target organs (including kidney, hematological system, fee, heart, digestive system and central nervous system). (2) Moderate to heavy SLE: refers to the involvement of important organs and affects their functions. Renal involvement: glomerulonephritis, acute progressive glomerulonephritis, nephrotic syndrome; hematologic involvement: hemolytic anemia, granulocytopenia, thrombocytopenia, thrombotic thrombocytopenic purpura; neurologic involvement: convulsions, impaired consciousness, coma, stroke, transverse myelitis, mononeuritis or polyneuritis, psychiatric symptoms, demyelination syndrome; digestive system involvement: intestinal obstruction, mesenteric vasculitis, acute pancreatitis; respiratory system involvement: alveolar hemorrhage, pulmonary hypertension, pneumonia, interstitial fibrosis, etc.; cardiovascular system involvement: pericardial tamponade, myocarditis, etc.; others: cutaneous vasculitis, severe skin lesions, myositis, etc. (3) Definition of lupus danger: acute life-threatening severe SLE is called lupus crisis, and the main clinical manifestations include: acute glomerulonephritis, severe central nervous system damage, severe hemolytic anemia, severe thrombocytopenic purpura, severe granulocyte deficiency, severe heart damage, severe lupus pneumonia or alveolar hemorrhage, severe lupus hepatitis, severe vasculitis, etc.  4. Treatment of light SLE (1) Glucocorticoids are not the first choice of treatment drugs in the treatment of light SLE; (2) Consider the application of non-steroidal anti-inflammatory drugs and anti-malarials, etc. After the treatment is ineffective, glucocorticoids can be considered; (3) Glucocorticoids can be used for a short period of time in the treatment of skin mucosal lesions, but the use of strong hormonal topical drugs should be avoided in the face, and even if used, it should not exceed 1 week. (4) Hormones (prednisone <10mg/d or methylprednisolone <8mg/d) help control the disease and usually have fewer side effects.  5. Treatment of moderately active SLE (1) Treatment of moderately active SLE is generally divided into 2 phases, namely induction of remission and maintenance therapy, and glucocorticoid combined with immunosuppressive therapy is recommended. (2) Induction remission therapy: the dosage of glucocorticoid is usually prednisone 0,5-1mg/kg/d, taken in the morning, and can be taken in divided doses if acute activities such as persistent hyperthermia need to be controlled. After d, the rate of reduction will be adjusted slowly according to the disease. If the condition allows, the dose of maintenance treatment: prednisone <10mg/d. In the process of dose reduction, if the condition is unstable, the original dose can be temporarily maintained unchanged or treated with immunosuppressive drugs.  6.Treatment of heavy SLE patients (1) Treatment of heavy SLE patients especially emphasizes individualized protocols and requires concurrent use of other immunosuppressive drugs; (2) Treatment of heavy SLE is also divided into 2 phases, namely induction of remission and maintenance therapy. Induction of remission: the glucocorticoid dosage is usually chosen as the standard dose of prednisone 1mg/kg/d (methylprednisolone 0,8mg/kg/d), taken at dawn, and intravenous methylprednisolone 500-1000mg for 3 consecutive days of shock therapy can be chosen for type III/IV lupus nephritis. Maintenance treatment: 2 weeks after the condition is stabilized or within 8 weeks of treatment, the dose can be reduced slowly at a rate of 10% every 1-2 weeks to 0.5mg/kg/d of prednisone, and then the rate of reduction will be adjusted slowly according to the condition.  7. Treatment of lupus crisis (1) For lupus crisis, high-dose methylprednisolone shock therapy is usually needed to help patients pass the crisis; (2) High-dose methylprednisolone shock therapy means: methylprednisolone 500-1000mg once a day, add 5% glucose 100-250ml, slowly infuse intravenously for 1-2h, and apply for 3 consecutive days as a course of treatment. If the lupus crisis is still not controlled, it is feasible to give oral prednisone 0,5-1mg/kg/d (methylprednisolone 0,4-0,8mg/kg/d) after shock for about 4-8 weeks; after the disease is controlled, the glucocorticoid should be gradually reduced until the minimum dose is reached to control the disease, in order to avoid serious adverse effects caused by long-term use of glucocorticoids. (4) For severe neuropsychiatric lupus, including transverse myelitis, dexamethasone 10 mg and/or methotrexate 10 mg intrathecal injection can be applied when central infection is excluded (intrathecal injection is recommended once a week for a total of 3-5 times); (5) Methylprednisolone shock therapy can only resolve the symptoms in the acute phase, and subsequent treatment must continue to apply glucose corticosteroids and used in conjunction with other immunosuppressive agents. The side effects of hormones should be closely observed before, during and after high-dose shock therapy (closely observe the occurrence of complications such as infection, gastrointestinal bleeding, diabetes mellitus and femoral head necrosis).  8. Contraindications to pregnancy in SLE patients (1) severe lupus flares within the past 6 months, such as active lupus nephritis; (2) severe pre-eclampsia or HELLP syndrome despite treatment; (3) severe pulmonary hypertension (expected pulmonary artery systolic pressure value >50 mmHg or symptoms); (4) severe restrictive lung disease (exertional spirometry <1l< span=" ">); (5) chronic renal failure (creatinine level >247, 8mmol/L).  9, use in pregnant patients (1) before pregnancy, in the absence of significant organ damage, stable disease for 1 year or more, cytotoxic immunosuppressants discontinued for six months, hormones only when maintained with prednisone <10mg/d, does not affect pregnancy. (2) Corticosteroids should be used cautiously during pregnancy, applying the lowest effective dose, preferably prednisone <20mg/d (methylprednisolone <16mg/d); if the disease is active, severe activity is life-threatening then immediate termination of pregnancy is required; if pregnancy can continue after disease assessment, increase the hormone dose as appropriate (prednisone <30mg/d or methylprednisolone <24mg/d), prednisone is recommended Prednisone, prednisolone, methylprednisolone, dexamethasone and betamethasone are not recommended; use of hormones during the third trimester may increase the risk of fetal cleft lip and palate, therefore medium to high doses of hormones during the third trimester are not recommended; patients treated with long-term corticosteroids should be treated with emergency doses at delivery; intravenous methylprednisolone shocks may be considered in case of relapse; in the second trimester, dexamethasone may be used to promote fetal lung maturation Dexamethasone.  10. Use in lactating patients (1) During lactation, methylprednisolone is relatively safe at 16-24 mg/d. It is recommended to take glucocorticoids for more than 4 hours before breastfeeding. Supplementation with vitamin D and calcium until the end of the lactation period. (2) Management of congenital heart block in fetal and neonatal lupus syndrome: The most common cardiac manifestation of neonatal lupus syndrome is congenital conduction block, which tends to have a high morbidity and mortality. Fluorinated hormones (dexamethasone and betamethasone) improve the survival of fetuses with congenital heart block when administered transplacentaally. However, these drugs also carry a higher risk of intrauterine growth retardation and preterm delivery; (3) prevention of miscarriage due to antiphospholipid antibodies: antiphospholipid antibodies are present in about 1/4-1/2 of SLE patients, and the main problem facing pregnancies in SLE patients exposed to antiphospholipid antibodies is the increased risk of miscarriage. Although anticoagulation therapy is the primary means of prevention, and the combination of glucocorticoids and aspirin can reduce the risk of miscarriage, the incidence of maternal complications is high.  11. Adverse effects of glucocorticoids (1) The course of hormone therapy for SLE is long and care should be taken to protect the hypothalamic-pituitary-adrenal axis. It is recommended to avoid the use of long-acting and super-long-acting hormones such as dexamethasone, which has a strong effect on the HPA axis. (2) Long-term or high-dose or irregular use of glucocorticoids can induce and aggravate infections, lead to osteoporosis and aseptic necrosis of the femoral head, peptic ulcers, neuropsychiatric disorders, hypertension, diabetes mellitus, hyperlipidemia, sodium retention, hypokalemia, glaucoma, Cushing's syndrome and some other adverse reactions, and even lead to death of patients in serious cases; (3) Adverse reactions of hormone application are related to their dose and course of treatment (3) The adverse effects of hormone application are related to the dose and duration of treatment, and need to be regularly observed and evaluated to ensure the efficacy and safety, and to improve the survival rate and prognosis of SLE treatment. (The above content is exclusively produced by China Rheumatology Public Forum)